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Background: Dyslipidemias and essential fatty acid deficiencies (EFADs) are well established complications of cystic fibrosis (CF). In the general population, a diet high in saturated fat is associated with hyperlipidemia and greater risk of cardiovascular disease and type 2 diabetes. Increasing life expectancy in CF brings concern about the risks of the "legacy" high-fat CF diet. The impact of CFTR modulators on CF-related dyslipidemia and EFAD is not known. Previous studies reported dyslipidemia in people with CF (PwCF) using traditional lipid measures. This study aimed to evaluate the lipoprotein and fatty acid profiles in children and adolescents with CF and to correlate biochemical results with clinical and molecular findings. Plasma and red blood cell (RBC) samples were studied to compare the ability of each method to identify EFAD markers. Method(s): Blood samples (n = 171) were obtained from 142 (78 female) children with CF aged 9.8 +/- 4.7 (range 4 months to 18 years) during routine laboratory draws at pediatric CF center clinic visits. Pancreatic insufficiency was present in 92% and glucose intolerance or diabetes in 14%. Body mass index percentile (BMI%ile) for age z-scorewas 0.23 +/- 0.89 (range -2.4-2.6). F508del mutation was homozygous for 56% and heterozygous for 41%. CFTR modulator therapy had been initiated 3 or more months before for 62% of samples. Sample collection began in September 2019, paused during the COVID-19 pandemic, and resumed in July 2021. An accredited, regional laboratory with expertise in fatty acid analysis processed all samples. Serum was separated and refrigerated for lipoprotein analysis, plasmawas separated and frozen, and RBCs were washed and frozen for fatty acid analysis. Nuclear magnetic resonance lipoprotein assayswere conducted to determine particle number and size of lipoprotein classes. Triglyceride, total cholesterol, and high-density lipoprotein cholesterol (HDL-C) were measured directly (Roche). Low-density lipoprotein cholesterol (LDL-C) and very low-density lipoprotein cholesterol (VLDL-C) were calculated. To correlate laboratory results with clinical findings, medical records were reviewed, and a CF clinic dietitian conducted 24-hour dietary recalls concurrent with study labs. Result(s): Of PwCF homozygous F508del/F508del, 43% tested positive for EFAD biomarkers (RBC linoleic acid, RBC mead acid, RBC triene/tetraene ratio), compared with 13% of PwCF heterozygous F508del ( p <=0.01) (Figure 1). There was no significant difference in concentrations of fatty acid and EFAD biomarkers between those who had or had not initiated CFTR modulator therapy. Lipoprotein abnormalities were identified in 69% of samples with low HDL-C and 39% with large HDL-C, 87% with large VLDL-C particle size and 52% with large VLDL-C particle number, and 5% with high LDL-C or small LDL-C particle numbers. High total cholesterol was found in 15% and high triglycerides in 17%. HDL-C was low in 24%, and 3% had high LDL-C. (Figure Presented) Figure 1. Differences in concentrations of red blood cell (RBC) linoleic and mead acids and triene/tetraene (T/T) ratio between F508del homozygous and F508del heterozygous individuals Conclusion(s): Despite clinical advances and use of CFTR modulator therapy, EFAD remains prevalent and underrecognized in the pediatric CF population. Of PwCF, those homozygous for f508del may have a higher risk of EFAD. Limitations of this study (four different CFTR modulator therapies and small sample sizes in each group) may have precluded significant findings for EFAD and lipid profiles, but PwCF receiving modulator therapy appear to have healthier lipid profiles than those not receiving therapy. Lipids and fatty acid are not routinely evaluated in PwCF, but evaluation should be included in the standard of care for timely dietary interventionsCopyright © 2022, European Cystic Fibrosis Society. All rights reserved
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Background: Since 2015, the Cystic Fibrosis Foundation (CFF) has collected and reported experience-of-care (XoC) data. Data collection was discontinued with the onset of the COVID-19 pandemic in 2020. In 2021, CFF convened a steering committee of a person with cystic fibrosis (CF), caregivers, and clinicians to develop a XoC survey to help understand and improve the XoC at CFF accredited programs. Method(s): Using prior CFF patient and family XoC surveys (2015-2020 pediatric and adult versions) [1,2] and a telehealth survey created in 2020 [3], draft pediatric and adult versions of the survey were developed. The steering committee and CFF leaders conducted three rounds of reviewand revision. After the surveys were professionally translated into Spanish, and the CFF Spanish Speakers Committee reviewed them, the surveys were programmed into Qualtrics for data collection. The data collection process was piloted with selected programs before a national launch. Result(s): Pediatric and adult surveys were developed in English and Spanish. The surveys cover in-person and telehealth (phone/video) visits and visits that are a mix of in-person and telehealth. The topics include interactions with care team members, relationship-centered care, care planning, shared decision-making, overall quality of care, race and ethnicity, gender identity, infection, prevention and control, quality of the virtual connection, and experience with remote monitoring. People with CF (PwCF) and their families are invited to complete a survey once every 6 months by text or email. PwCF and family contact data (email and mobile phone number) are stored in CFF's CFSmartReports Patient and Family Tool. After a clinic visit, contact data are electronically transferred to the Qualtrics platform to trigger a survey invitation. Responses are anonymous and reported back to programs via an electronic dashboard in near-real time. The data collection process was tested with three pediatric and three adult care programs for 3 weeks before the national launch on October 25, 2021. More than 2,000 PwCF and their families have completed a survey. Conclusion(s): The new XoC surveys offer PwCF and their families an opportunity to share feedback about their in-person and virtual care experiences. Efforts are underway to create a national report for dissemination and to engage programs with the data reported in their dashboards to celebrate what PwCF and their families appreciate about their care and to work together with them to improve gaps.Copyright © 2022, European Cystic Fibrosis Society. All rights reserved
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Background: Workflow for cystic fibrosis (CF) outpatient care changed rapidly on 3/11/20 when the World Health Organization declared COVID-19 outbreak a global pandemic. Our goal was to provide safe, quality patient care while following institutional mandates. Methods: Within weeks, the majority of CF appointments were transitioned to virtual visits. Three new visit types were implemented: Telehome (TH), non-face-to-face phone calls, and essential in-clinic. A few virtual platforms were trialed based on institution compliance. TH equipment (microphones, cameras and second computer monitors) was quickly ordered and installed. The medical assistant initiated the TH visit by calling the family, documenting reported home measurements (height, weight), helping them log into the virtual platform and providing technical support. The pharmacist joined to review medications and help with assistance programs. Next, the CF provider met virtually with the patient and family. Finally, the social worker, dietitian and nurse joined or followed up via phone or email. The provider and multidisciplinary team communicated throughout the virtual clinics utilizing a workplace communication platform (Microsoft Teams®). This platform allowed all team members to give input and eventually, operated as the virtual team board, so multiple team members could synchronously join the visit. Gastroenterology, endocrinology and child psychology scheduled separate virtual visits. Respiratory therapy (RT) changed all annual respiratory assessments (ARA) and new treatment education to virtual visits. Physical therapy (PT) created exercise videos that were shared with families. Results: Virtual CF visits began 3/31/20;virtual RT appointments began 4/8/20. Below are TH visit counts from 3/31/20-5/29/20 (See Table). In addition, 12 patients were seen for an in-clinic essential visit;3 patients were hospitalized. Discussion: Providing care to patients and families during a pandemic involved creativity, flexibility and modification of previous workflows. The relaxation of billing codes and insurance regulations allowed for the swift implementation of TH visits. Technology allowed for regular communication between CF team members. Families were able to complete the ARA without having to bring respiratory equipment to the clinic appointment. The RT was able to evaluate the equipment and working condition, assess proper airway clearance technique and provide the necessary durable medical equipment. Conclusion:The greatest barrier to implementation of TH visits was identifying the most reliable virtual platform that allowed integration of the multidisciplinary team and was simple to use for patients and families. Telehome visits and ARAs may continue to be offered post-pandemic for some patients, some of the time.
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Background: On March 11, 2020 the World Health Organization declared the outbreak of coronavirus disease (COVID-19) a global pandemic. This significantly changed our operations and modes of communication. Our goal was to provide timely, up-to-date, and streamlined information and maintain high quality medical care and emotional support for patients, families, and the CF care team. Methods: Multiple means of communication were utilized to meet the needs of the various groups: Patients/Families: A central, secure email address was created for the CF center. Emails were collected utilizing a master list. On March 12, 2020, the first email was sent;a weekly newsletter followed. Emails included updates on the COVID-19 pandemic, recommendations for minimizing the risk of contracting the virus, information on rapid changes to in-clinic visits and new telehealth visit process, and other resources from the CF team (exercise videos, nutrition materials, food pantry/WIC updates, patient assistance programs, Healthwell funds information, scholarship resources, and wellness links). CF care team members created short videos with the theme, “What Brings Me Joy,” to demonstrate the power of reframing and focus on shared humanity. Videos were posted on a private social media page for all to view. Families were encouraged to make and share videos as well. The local Cystic Fibrosis Foundation (CFF) chapter hosted monthly virtual town hall meetings, during which the CF medical director and care team answered questions submitted by patients and families. Staff: Team members began working from home the week of March 16, 2020. Virtual huddles with the CF team were initiated March 25, 2020 and occurred every other weekday. Huddles began with a guided meditation, followed by open-forum staff check-ins, COVID-19 updates/questions and process updates. Huddles transitioned to twice weekly with a planned agenda on April 13, 2020. A “WhatsApp” group chat was established for CF team members for quick sharing of non-patient related information, announcements, uplifting photos, words of encouragement, and jokes. Results: Weekly newsletter was sent to CF patient/family email list starting March 12, 2020. The email list started with 274 addresses;73 emails were added and/or corrected in the following weeks. Information allowed for factual, up-to-date communication regarding the pandemic, clinic visits, and hospital policies. Informal feedback from parents was overall positive, however, families did report a degree of information overload. Communication through virtual meetings, apps, and videos allowed for professional and personal interactions and provided a source of unity for our care team, despite physical distancing. Conclusion: Weekly newsletter communication from the CF care team may transition to a monthly and then a quarterly newsletter when the pandemic resolves. There is potential for ongoing collaboration with the local CFF chapter to continue holding town hall meetings in the future. The bond amongst the CF care team remains strong, as we care for our patients and support each other.